Evaluate for Guillain-Barré Syndrome in Patients with Acute Paralysis or Respiratory Failure and Areflexia
Jose I. Suarez MD
Guillain-Barré syndrome (GBS) represents the most common cause of acute flaccid paralysis in otherwise healthy adults in the United States. GBS is more frequent in men as compared with women of all ages. There is a slight increase in the incidence of GBS in younger adults and in those older than 50 years of age.
Most patients will complain of a flulike illness about 1 to 3 weeks prior to initiation of symptoms. Other possible factors that may be associated with Guillain-Barré syndrome include infectious agents such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, influenza virus, and human immunodeficiency virus; recent surgery; immunization; and systemic illnesses such as Hodgkin’s disease and systemic lupus erythematosus. How these factors trigger or lead to Guillain-Barré syndrome is still unclear. However, mounting basic and clinical evidence supports an immune-mediated attack on the peripheral nervous system. Pathologic evaluation of peripheral nerves of GBS patients reveals perivascular infiltration by mononuclear cells along with demyelination and axonal loss.
Clinical Presentation
The major clinical symptoms and signs include progressive limb weakness and areflexia (Table 208.1). Such weakness usually presents over a period of several hours to days. About half of patients will have ascending involvement of lower extremities followed by upper extremities. However, a variant presenting with descending paralysis starting in cranial nerves or upper limbs can be seen in about 14% of cases. Limb weakness does not usually progress beyond 4 weeks and half of the patients experience a nadir within 2 weeks. Frequently patients complain of limb paresthesias and dysesthesias concomitantly or preceding limb weakness that is sometimes accompanied by lower back pain. On physical examination practitioners encounter areflexia or severely decreased myotatic reflexes and usually symmetric limb involving both distal and proximal muscles. On exam, the most common cranial nerves involved are facial, glossopharyngeal, and vagus nerves. Thus patients will have facial weakness (usually bilateral) and oropharyngeal
dysfunction manifested by dysphagia and dysarthria. Compromise of the cranial nerves innervating the extraocular muscles (i.e., III, IV, and VI cranial nerves) is less common and is usually part of a Guillain-Barré syndrome variant also known as Miller Fisher variant. The latter
can present with ophthalmoparesis or ophthalmoplegia and areflexia followed by respiratory failure. The progression and severity of GBS are variable with 33% of patients requiring mechanical ventilation.
dysfunction manifested by dysphagia and dysarthria. Compromise of the cranial nerves innervating the extraocular muscles (i.e., III, IV, and VI cranial nerves) is less common and is usually part of a Guillain-Barré syndrome variant also known as Miller Fisher variant. The latter
can present with ophthalmoparesis or ophthalmoplegia and areflexia followed by respiratory failure. The progression and severity of GBS are variable with 33% of patients requiring mechanical ventilation.
TABLE 208-1 DIAGNOSTIC CRITERIA FOR GUILLAIN-BARRÉ SYNDROME | ||||||||||||||||
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